Key result
Degos' disease manifests as calcific constrictive pericarditis with previously unreported radiographic pericardial calcification.
Case Report (n=1)
This case report describes the first known instance of radiographically demonstrable calcific constrictive pericarditis in a patient with Degos' disease.
Alerts clinicians to rare pericardial involvement in Degos' disease; extends the known phenotype but remains hypothesis-generating.
A 31-year-old woman presented with symptoms and signs of constrictive pericarditis. She had a history of Degos' disease, a rare disorder characterized by skin and bowel lesions thought to be secondary to vasculitis. A chest roentgenogram showed extensive calcification of the pericardium. Although pleural and pericardial involvement has been reported in this disease, constrictive pericarditis is most unusual, and radiographically demonstrable calcification of the pericardium has not been reported previously.
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McLoud et al. (1978) conducted a case report in Calcific constrictive pericarditis in Degos' disease (n=1). Degos' disease was evaluated. A 31-year-old woman with Degos' disease presented with calcific constrictive pericarditis, an unusual manifestation with previously unreported radiographically demonstrable pericardial calcification.
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